Pancoast Tumor

• A Pancoast tumor is a lung cancer located at the very top (apex) of the lung.
• The Pancoast tumor is defined by its location. Pancoast tumors are sometimes referred to as superior sulcus tumors.
• Most Pancoast tumors are non-small cell lung cancers (NSCLC); a few are small cell lung cancers (SCLC).
• Pancoast tumors spread to the tissues around them, including the neck and chest nerves, ribs, and vertebrae.
• Symptoms of this disease may be referred to as Pancoast syndrome and include pain in the shoulder, inner side of the arm, and hand.
• Pancoast tumors rarely produce symptoms related to the lungs themselves, such as chest pain or cough.
• Treatment for Pancoast tumors involves a combination of surgery, chemotherapy, and radiation.

Pancoast tumors are lung cancers that form at the extreme apex (very top; plural=apices) of either the right or left lung in the superior sulcus (a shallow furrow on the surface of the lung). Because of their location in the top of the lung, they invade adjoining tissue and produce characteristic symptoms. They form an abnormal patch of tissue over the lung apex and principally involve the chest wall structures rather than the underlying lung tissue. They invade the following structures:

• Lymphatics (small, thin vessels that carry lymph fluid through the body)
• Lower roots of the brachial plexus (a complex network of nerves that is formed chiefly by the lower four cervical [neck] nerves and the first thoracic [chest] nerve)
• Intercostal nerves (nerves that lie between a pair of adjacent ribs)
• Stellate ganglion (a mass of nerve tissue containing nerve cells that form an enlargement on a nerve or on two or more nerves at their point of junction or separation)
• Sympathetic chain (either of the pair of ganglionated lengthwise cords of the sympathetic nervous system that are situated on each side of the spinal column)
• Adjacent ribs
• Vertebrae

Carcinomas (cancerous tumors) in the superior pulmonary sulcus produce the Pancoast syndrome, which is characterized by pain in the shoulder and along the inner side of the arm and hand. Pancoast tumors tend to spread to the tissue surrounding them in the early stage of the disease. As long as the cancer has not metastasized (spread) and involved the regional lymph nodes (small, bean-shaped structures found throughout the body), these tumors can be successfully treated.

It is important to note that a Pancoast tumor is defined by its location in the top portion of the lung. The term does not refer to a specific subtype of lung cancer such as small cell lung cancer (SCLC) or non-small cell lung cancer (NSCLC). Most Pancoast tumors are NSCLC of the squamous cell carcinoma type, although adenocarcinomas and large cell carcinomas can also form in the lung apex and be referred to as Pancoast tumors. A small percentage (3%-5%) of Pancoast tumors are SCLC.

The risk factors for almost all lung cancers are similar. These include the following:

• Smoking
• Secondary smoke exposure
• Prolonged asbestos exposure
• Radon exposure
• Exposure to industrial elements (for example, gold, nickel)

Although a Pancoast tumor is a lung tumor, it rarely causes symptoms that are typically related to the lungs (for example, cough and chest pain). Instead, it causes symptoms (Pancoast syndrome) related to invasion of surrounding tissues in its unique location at the apex of the lung. The initial symptom is pain in the shoulder radiating to the inner part of the scapula (large, triangular, flattened bone that lies over the ribs on the back).

The pain may later extend to the inner side of the arm, elbow, and the pinky and ring fingers.

The associated pain is severe and constant, often requiring narcotic pain medications for relief. The affected person usually needs to support the elbow of the affected arm in the opposite hand to ease the tension on the shoulder and upper arm.

The hand, arm, and forearm may weaken, atrophy (degenerate or shrink from disuse), or develop paresthesia (a sensation of pricking, tingling, or creeping on the skin). Edema may develop if blood vessel compression occurs.

If the tumor extends to the sympathetic chain (a series of ganglia [masses of nerve cells] that run parallel to the vertebrae) and stellate ganglion, Horner syndrome may develop on the face and hand of one side of the body. Horner syndrome is characterized by drooping eyelids (ptosis), absence of sweating (anhidrosis), sinking of the eyeball (enophthalmos), and excessive smallness or contraction of the pupil of the eye (miosis).

In as many as 10%-25% of people with Pancoast tumor, compression of the spinal cord and paraplegia (paralysis of the lower half of the body with involvement of both legs) develop when the tumor extends into the intervertebral foramina (opening between two vertebrae).

The symptoms of Pancoast syndrome can also be caused by other cancers or other conditions that lead to growths in the same area of the chest. While lung cancer is the typical cause of Pancoast syndrome, the characteristic symptoms may be causes by other conditions that include thyroid carcinoma, lymphoma, infections, aneurysms, cervical rib syndrome, and other diseases.

Pancoast tumors are typically treated by a multidisciplinary team that includes thoracic surgeons or surgical oncologists, medical oncologists, and radiation oncologists.

The blood exam for persons with Pancoast tumor is not specific, and the results are not diagnostic.

• Chest X-ray film
  • In the early stages, Pancoast tumors are difficult to detect on chest X-ray films because the top of the lung is located in an area of the body that is difficult to visualize clearly on an X-ray film. Shadows (visual interference from nearby body structures) that lie over the lungs make the image on the X-ray film unclear. Many patients end up consulting orthopedic surgeons and neurologists before a definitive diagnosis is made. An apical lordotic view ordered with the chest X-ray may visualize the problem in patients with suspicious symptoms.
  • A chest X-ray film may reveal asymmetry of the tops of the lungs in the form of a small, uniform patch of tissue or pleural thickening (a thickened, abnormal area of tissue) on the apex of one lung. The X-ray film may show a thin plaque (patch of abnormal tissue) at the lung apex in the area of the superior sulcus, or the X-ray film may reveal a large mass, depending on the stage when it is first diagnosed.
  • The plain chest X-ray film may show that the tumor has invaded one or more ribs or parts of the vertebrae. Bone destruction of 1-3 of the back ribs may sometimes be visible on the X-ray film.
  • Enlargement of the mediastinum (area of the chest between the lungs) may be visible on the X-ray film.
• CT scan of the chest: A CT scan helps the doctor determine if the tumor has invaded the brachial plexus, chest wall, mediastinum, vertebra, or a combination thereof. CT images can also reveal involvement of the vena cava, trachea (windpipe), and esophagus (food pipe). Contrast CT scanning, with the help of an injected, radioactive dye that is visible on the scan, is useful for assessing if the tumor has involved the blood vessels under the clavicle (collarbone).
• MRI of the chest: MRI findings are more accurate than CT scan findings in identifying the extent of the tumor involvement. An MRI can also better assess the tumor’s invasion of nearby structures and vertebral bodies and if cord compression is occurring. However, MRI findings have no advantage over CT scan findings in the evaluation of the mediastinum. In fact, CT scan findings are much better than MRI for assessing the mediastinum to determine if the tumor has involved the lymph nodes.
• Arteriogram or a venogram (an X-ray taken after the injection of an opaque [nontransparent] substance into a vessel): Rarely, the Pancoast tumor involves the artery or the vein under the clavicle; therefore, an arteriogram or a venogram may be helpful.
• Bronchoscopy (using a tubular, illuminated instrument for inspecting the airways of the lung) helps evaluate the tracheal and bronchial cavities; however, because most Pancoast tumors form on the periphery of the lung, bronchoscopy does not usually help the doctor make a diagnosis.
• Biopsy (removal of cells for examination under a microscope): Following a biopsy, the doctor can make a diagnosis in 95% of persons based on results from percutaneous (through the skin) needle biopsy, either with the guide of fluoroscopy (procedure using an X-ray machine and a fluorescent screen to view inside the body) or with CT-guided procedures.
• Although most patients can be correctly diagnosed based on clinical and radiological findings (chest X-ray, CT, MRI) alone, open biopsy of the tumor for confirmation may be performed through a supraclavicular (above the collarbone) incision. A definite diagnosis is important before proceeding with treatment of the Pancoast tumor. Results from a needle biopsy are also useful in determining the cell type prior to treatment. Even though clinical diagnosis is relatively simple, performing a tissue biopsy is always necessary. The significance of an accurate diagnosis of cancer cannot be overstated.

Exams to determine spread

• In patients with an abnormal neurologic examination not explained by the local and regional involvement by the Pancoast tumor, CT or MRI of the brain is recommended in the initial evaluation because distant metastases to the brain are not infrequent, and diagnosis of these is necessary for staging.
• Mediastinoscopy: This procedure is performed to determine the extent the tumor has spread into the mediastinum. It is a procedure in which a tube is inserted behind the breastbone through a small cut at the lowest part of the neck. Samples of lymph nodes are taken from this area to look for cancer cells.
• Staging of the cancer is based on scalene (neck muscle) node biopsy results from palpable (capable of being felt) nodes or mediastinoscopy findings.
• Bone scans are performed to determine if the cancer has spread.
• CT scans of the abdomen assess particularly the liver and adrenal glands and upper abdominal lymph nodes.
• Positron emission tomography (PET) scans (a nuclear imaging technique used to view body functions) in combination with CT scans of the chest and abdomen, may help identify involved lymph nodes and the distant spread of cancer.

Staging of Pancoast tumors is determined by TNM system according to the American Joint Committee on Cancer (AJCC). T describes the size and invasiveness and N describes the distribution of positive lymph nodes, while M designates the extent of distant metastases. Staging can help predict the patient is a candidate for surgery; for example, individuals with distant metastases are usually not surgical candidates.

The optimal treatment for Pancoast tumors has evolved. All lung cancer treatment continues to consider the patient's overall condition and what is specifically called performance status in making treatment decisions. The performance status compares the patient's condition to their pre-illness presumably normal activity levels. In patients with limited tumors and early Pancoast syndrome and good performance status, today the ideal treatment of even potentially operable cancers is a combination of cisplatin based chemotherapy and radiation.

This is then followed by restaging, and when appropriate, aggressive surgical resection of the entire lung and affected adjacent tissues followed by further postoperative chemotherapy. This approach can be curative in as many as half of patients followed for five years or more.

Alternative approaches in selected patients including surgical resection alone, preoperative radiation and surgery (curative in up to 30% without lymph node involvement), or just radiation alone can be considered in selected cases. The best results have been reported with combined multi-modality treatment.

The primary sites of failure after surgical resection with clear margins have been in the brain. Palliative radiation therapy for inoperable disease in patients with poor treatment outcomes can reduce pain. Palliative chemotherapy for disseminated disease today is appropriate in patients with good reaction to treatment and can prolong life and reduce symptoms. Steroids to reduce pressure on nerves can be helpful, as can aggressive symptom management with pain medications.

The most important preventive step is to avoid using tobacco products. Quitting tobacco can reduce the risk of lung cancer. For more information, see Cigarette Smoking.

Taking precautions to reduce exposure to harmful substances in the environment (for example, asbestos) can reduce the risk of developing lung cancer.

In the past, Pancoast tumors were considered inoperable and incurable because of their relative inaccessibility and extensive invasion of nearby tissues and structures. However, recent studies have shown that, in some persons, the tumor completely stops growing and the pain is gone. In addition, survival rates have improved. Chemotherapy and radiation can result in no evidence of viable cancer in the surgical specimen.

Clinical studies demonstrate that even preoperative irradiation alone in doses not strong enough to shrink the tumor (1) decreases the chance of recurrence (that the tumor will grow back), (2) prevents tumor cells from growing elsewhere in the body, and (3) increases the chance of survival compared with irradiation or surgical resection alone.

By surgically removing the lower trunk of the brachial plexus, the distribution of the ulnar nerve (nerve that runs along the inside of the arm and hand) is permanently damaged, but the person is not incapacitated. After surgery, Horner syndrome and anhidrosis (inability to sweat) develop following the removal of parts of the sympathetic nervous system. However, these do not disable the person. This type of surgery should be performed in a tertiary referral center.

Factors that are associated with a poor prognosis include Horner syndrome, mediastinal lymph node involvement, and an incomplete surgical removal of the tumor. To date, no patient with any of the above factors has survived for five years.

Factors that have a negative effect on outlook can be summarized as follows:

• Presence of Horner syndrome
• Involvement of mediastinal lymph nodes
• Involvement of supraclavicular lymph nodes
• Invasion of the tumor into areas of the vertebrae

A diagnosis of cancer can feel overwhelming. Interacting with people with a similar condition helps one deal with the uncertainties and challenges that cancer brings.

The following organizations can help you with support and counseling:

• The AMC Cancer Information and Counseling Line provides current medical information and counseling for cancer issues. Tel: (800) 525-3777.
• The Alliance for Lung Cancer Advocacy, Support, and Education operates a national "phone buddies" program, in addition to other services. Tel: (800) 298-2436, email: [email protected].
• The National Coalition for Cancer Survivorship is a survivor-led advocacy organization working exclusively on behalf of people with all types of cancer and their families.

American Cancer Society
(800) ACS-2345

American Lung Association
61 Broadway, 6th Floor
New York, NY 10006
(212) 315-8700, (800) LUNG-USA (586-4872)

National Cancer Institute, Cancer Information Service
NCI Publications Office
6116 Executive Blvd MSC8322
Suite 3036A
Bethesda, MD 20892-8322
(800) 4-CANCER (422-4237)
TTY (for deaf and hearing-impaired callers) (800) 332-8615

American Society of Clinical Oncology (ASCO)
225 Reinekers Lane
Suite 650
Alexandria, VA 22314
(703) 299-0150

U.S. National Institutes of Health, National Cancer Institute, Lung Cancer